Jain Ashokkumar B, Patil Vrishali, Sheikh Baber, Apostolakos Michael, Ryan Charlotte, Kashyap Randeep, Orloff Mark
Department of Surgery, Division of Abdominal Organ Transplant, Temple University Hospital, Philadelphia, PA 19140, USA.
Exp Clin Transplant. 2010 Mar;8(1):4-8.
Alpha 1 antitrypsin (A1A) is a 52 kD glycoprotein that is mainly synthesized in the liver. As a major protease inhibitor, it binds to and neutralizes neutrophil elastase, thereby limiting the damage to the normal tissues after an inflammatory response. A deficiency in A1A leads to end-stage liver disease, both in children and in adults. In addition, the deficiency also has a detrimental effect in the lungs of the adult population. Alpha 1 antitrypsin deficiency is corrected with hepatic replacement; however, the changes in pulmonary functions have not been studied before and after liver transplant. The purpose of this study was to observe the changes in the pulmonary functions of patients who underwent liver transplant for the treatment of A1A deficiency.
Nine patients underwent liver transplant for A1A deficiency. Seven patients (5 men, 2 women; mean age, 49.95 -/+ 7.09 years) had their pulmonary function tests available before the liver transplant (mean, 5.6 -/+ 3.4; range, 0.9-10.1 months) and after the liver transplant (mean, 30.3 -/+ 18.4, range 7.8-48.1 months) for analysis.
The mean, preliver, transplant, FEV1 was 2.69 -/+ 0.9 L, which was nearly unchanged after the liver transplant to a mean of 2.7 -/+ 1.2 L. During the mean total interval of nearly 3 years, an estimated decline of 250 mL in FEV1 was expected.
It appears from the results of our study that liver transplant probably prevented the progression of pulmonary disease in A1A-deficient patients. Further study and close, postliver, transplant follow-up is warranted to support our initial findings.
α1抗胰蛋白酶（A1A）是一种主要在肝脏合成的52kD糖蛋白。作为一种主要的蛋白酶抑制剂，它与中性粒细胞弹性蛋白酶结合并使其失活，从而限制炎症反应后对正常组织的损伤。A1A缺乏会导致儿童和成人出现终末期肝病。此外，这种缺乏对成年人群的肺部也有不利影响。α1抗胰蛋白酶缺乏症可通过肝脏替代来纠正；然而，肝移植前后肺功能的变化此前尚未得到研究。本研究的目的是观察因α1抗胰蛋白酶缺乏症接受肝移植患者的肺功能变化。
9例因α1抗胰蛋白酶缺乏症接受肝移植。7例患者（5例男性，2例女性；平均年龄49.95±7.09岁）在肝移植前（平均5.6±3.4；范围0.9 - 10.1个月）和肝移植后（平均30.3±18.4，范围7.8 - 48.1个月）进行了肺功能测试以进行分析。
肝移植前FEV1的平均值为2.69±0.9L，肝移植后几乎未变，平均值为2.7±1.2L。在近3年的平均总时间段内，预计FEV1会下降250mL。
从我们的研究结果来看，肝移植可能阻止了α1抗胰蛋白酶缺乏症患者肺部疾病的进展。需要进一步研究并在肝移植后进行密切随访以支持我们的初步发现。
